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Mixed Leiomyoma and Lymphangioma of the Epididymis
MIXED LEIOMYOMA AND LYMPHANGIOMA OF THE EPIDIDYMIS SAMUEL MALISOFF
AND
MILTON HELPERN
From The Beth David Hospital, New York, N. Y.
Primary tumors of the epididymis whether benign or malignant are very rare. In 1936 Thompson in a review of the literature and a report of 41 additional cases of tumors of the spermatic cord, epididymis and testicular tunic mentions only 3 cases of lymphangioma of the epididymis recorded in the literature. In 1939, 3 years later, Charache reviewing the literature on primary tumors of the epididymis listed 6 cases of lymphangioma including 1 of his own. Of the 54 cases of primary neoplasm of the epididymis which he recorded 22 were benign and of these 6 were lymphangiomata. Spivak in 1935 listed 11 cases of leiomyoma of the epididymis. Falconer reported a similar case in 1938, and Milner and Gilbert in 1939 described a case of bilateral leiomyomata of the epididymis in a 60 year old man in whom the Aschheim-Zondek test was negative. Halpert in 1941 reported a case of mixed leiomyoma of the epididymis as the first recorded example of such a tumor. The tumor he described was removed from a 60 year old Negro in whom swelling of the epididymis was noted 2 months after a suprapubic prostatectomy and bilateral vasectomy. The tumor was excised 3 months after the swelling was first noted and measured 3 by 2 by 1.5 cm. Grossly it had the appearance of a leiomyoma. Microscopically it was composed of a mixture of smooth muscle tissue and lymph capillaries imbedded in a connective tissue stroma. In this paper we are reporting a tumor of the epididymis similar to the one described by Halpert. CASE REPORT
A 57-year old white man, and a paper cutter by trade, stated that about 4 weeks before his admission to the Beth David Hospital on May 25, 1941, a ream of paper had slipped from his hands and struck him in the right groin. At that time, he experienced a sharp pain at that site, but the pain gradually subsided until 3 days before his admission to the hospital, when he became aware of a swelling of the right testicle accompanied by a sense of discomfort and dull pain in that organ. Physical examination disclosed a small, smooth, firm non-tender nodule about the size of a hazelnut in the right epididymis. The testicle was normal in size, shape and consistency. The spermatic cord on that side was also smooth and normal on palpation. There was no evidence of any inguinal hernia and no enlargement of the inguinal lymph nodes. The left testicle, epididymis and spermatic cord were normal. A preoperative diagnosis of possible traumatic right epididymitis was made, and the possibility of a neoplasm of the epididymis was also considered. On admission to the hospital the blood count revealed 4,950,000 erythrocytes, a hemoglobin of 97 per cent, 7900 leucocytes of which 66 per cent were poly104
MIXED LEIOMYOMA AND .LYMPHANGIOMA OF EPIDIDYMIS
105
morphonuclears, 3·2 per cent lymphocytes and 2 per cent monocytes. The Kline and Wassermann tests on the blood were negative. An Aschheim-Zondek test was carried out on a sample of urine obtained in the morning before operation on May 26. At the operation, the head of the epididymis, which was replaced by a tumor nodule, was resected by one of (S. M.) and submitted for rapid frozen section and histologic examination, and the diagnosis of a neoplasm was confirmed. An orchidectomy was then performed. When the epididymis was first removed, several cubic centimeters of clear straw-colored fluid were evacuated from the tunica vaginalis. Culture of this fluid was negative. The tunica vaginalis was not adherent to the testicle. A portion of the ductus deferens was also excised. Following the operation, the patient made an uneventful recovery and was discharged from the hospital ten days after admission. The Friedman modification of the Aschheim-Zondek test on the patient's urine, which was carried out on 1 rabbit, was positive, suggesting that the tumor of the epididymis would prove malignant. This was not borne out by the pathologic examination. Subsequently, three additional Aschheim-Zondek tests were performed during the post-operative period on June 4, June 30 and December 18, respectively. These subsequent tests were all negative. It is difficult to evaluate the results of the positive test in this case, although the animals used for all tests were obtained from a reliable source. Nevertheless, the possibility that a non-virgin female rabbit was injected, cannot be eliminated. The specimen first submitted for rapid frozen section was a globular tumor mass, measuring 18 mm. in diameter, replacing the head of the epididymis. The tumor was smooth, firm and elastic, and encapsulated. The resected surface by which it was attached to the pole of the testis measured 18 mm. in diameter. On section, the cut surface of the tumor nodule was gray in color and trabeculated, in appearance very much like that of a leiomyoma. The body and tail of the epididymis and the testis were submitted separately; the former measured 2 cm. in length and 5 mm. in diameter. The testis was normal in size and measured 4.3 by 3.2 by 2.8 cm. The tunica albuginea was smooth and beneath the site of the resected tumor nodule it was slightly thickened. There was a thin layer of freshly extravasated blood in the testicular tissue beneath this portion of the tunica albuginea. The appendices of the epididymis and of the testis were not found. Section of the testis revealed a normal lobular pattern. The structures in the spermatic cord were normal. Sections of the tumor nodule replacing the head of the epididymis revealed an encapsulated overgrowth of interconnecting fascicles of smooth muscle fibers intermingled with collagen fibers. This fibromuscular stroma was interspersed unevenly with many small closely placed, variable-sized clear spaces, separated from each other by thin delicate cytoplasmic walls containing flattened nuclei (fig. 1). At first glance, the pattern of these clear spaces suggested adipose tissue. On close examination, some of these spaces were found to contain small numbers of mononuclear leucocytes. Frozen sections of the tissue stained with Sudan III did not reveal any fat in these spaces, many of which were quite
106
SAMUEL MALISOFF AND MILTON HELPERN
large. '\¥here these spaces were numerous and large, the tissue had a honeycombed appearance. In other areas, where the vascular spaces were smaller, the supporting reticulum and endothelial lining cells were more readily made out. Some of the vascular spaces were formed intracellularly, first appearing as vacuoles in the cytoplasm of the endothelial cells. In such cells, the nuclei were more vesicular in character. Connective tissue reticulum was variable in amount and there were small scattered interstitial foci of lymphocytes. The tumor was enclosed in a thin fibrous tissue capsule. The fibromuscular charac-
FIG. 1. X 60
ter of the stroma was confirmed by the Van Gieson stain. No recognizable ducts or other tissue of the epididymis were found in any of the sections of the tumor. Sections of the testis beneath the site where the tumor of the epididymis was removed revealed slight fibrous thickening of the tunica of the tunica albuginea, and slight fresh hemorrhage beneath the tunica incident to the operation. The lymph vessels in the tunica are slightly dilated. The seminiferous tubules were fairly well preserved and active spermatogenesis was found in many of the
MIXED LEIOMYOMA AND LYMPHANGIOMA OF EPIDIDYMIS
107
tubules. Some atrophic and some dilated tubules were also found. The lymph vessels in the interstitium of the testis were dilated. There was no evidence of any neoplasm within the testis. Sections of the body and tail of the epididymis distal to the tumor revealed ·well-preserved ducts lined by ciliated epithelium. Many of these ducts contained spermatozoa. The lymph spaces in the epididymis were also slightly dilated, especially in the periphery, and some of the ducts of the epididymis were dilated.
Fm. 2. X 200
Diagnosis: The neoplasm replacing the head of the epididymis is most adequately diagnosed as a mixed leiomyoma and lymphangioma. DISCUSSION
Several papers describing cases of lymphangioma of the epididymis discuss the various theories of the histogcnesis of the tumor. A mechanical theory assumes that the lymphangioma occurs as a result of an obstruction to the flow of lymph resulting from inflammation of the lymphatics and lymph nodes,
108
SAMUEL MALISOFF AND MILTON HELPERN
:alteration in the perilymphatic tissues or obliteration of lymph channels as the result of traumatic injury, surgery or thrombosis. However, in none of the cases observed have such mechanical factors been satisfactorily demonstrated nor has there been any clinical evidence of lymph stasis. The heteroplastic theory, which assumes an origin of the elements of a lymphatic tumor from other types of tissue by metaplastic transformation, does not have much evidence to support it. The theory which is most generally accepted considers the lymphangioma to be a new growth derived from pre-existing lymphatic tissues. It is also possible that the lymphangioma has an embryonal basis for its origin. This possibility must certainly be considered in the present case because of the intimate association of the lymphangiomatous elements with the leiomyomatous portions of the tumor. Despite the history of trauma elicited in this case the pathologic findings do not lend any support to the theory that trauma is a factor in the causation of the tumor. There is nothing in the gross or histologic findings to indicate anything of a traumatic character in and around the site of the lesion. The tumor itself does not contain any elements that one would expect to encounter during an exaggerated reparativc process. There is no evidence of any old hemorrhage or scar tissue which might be interpreted as a residue and as an indicator of a previous traumatic injury. Except for the lymphangiomatous component of the tumor, the growth in no way differs from that of the ordinary leiomyoma so frequently encountered in the uterus and not infrequently found in the gastro-intestinal tract, sites where trauma has never been considered a causal factor. The positive Aschheim-Zondek test obtained preoperatively in 1 rabbit is difficult to evaluate. The tumor does not contain any elements comparable to those found in tumors which do give rise to the gonadatropic hormone. The possibility that the rabbit used was not a virgin cannot be eliminated. In the case reported by Milner and Gilbert the Aschheim-Zondek test was negative. SUMMARY
A second case of mixed leiomyoma and lymphangioma of the epididymis is reported. The pathologic findings, despite the history of a traumatic injury to the groin 4 weeks before the recognition of the tumor, do not suggest that trauma was a factor in the development of the tumor.
5 W. 86th St., New York, N. Y. REFERENCES CHARACHE, H.: Lymphangioma of the epididymis. Review of primary tumors of epididymis. Urol. and Cutan. Rev., 43: 663, 1939. FALCONER, B.: Zur Kenntniss der primaren N ebenhodengeschwulste. Ztsch. f. Krebsforsch., 48: 243, 1938. HALPERT, B.: Mixed leiomyoma and lymphangioma of the epididymis: report of a case. J. Urol., 45: 536, 1941. MARCANDIER ET THOMAS: Lymphangioma of the epididymis. Bull. de L'assoc. franc. p. l'Etude du cancer, 19: 126, 1930.
MIXED LEIOMYOMA AND LYMPHANGIOMA OF EPIDIDYMIS
109
MILNER, W. A., AND GILBERT, J.B.: Unique case of bilateral leiomyomata of epididymis. J. Urol., 42: 619, 1939. RIGANO-lRRERA, D.: On a case of simple circumscribed lymphangioma of the tail of the epididymis. Arch. Ital. di chir., 13: 552, 1925. ScALFr, A.: Cases of simple lymphangioma of epididymis. Boll. d. soc. med.-chir., Pavia, 49: 1053, 1935. ScALFI, A.: Benign tumors of the epididymis, Ann. ital. di chir., 16: 81, 1936. SPIVAK, A. H.: Leiomyoma of epididymis. Report of a case and review of literature. J. Urol., 34: 122, 1935. THOMPSON, G. J. : Tumors of the spermatic cord, epididymis and testicular tunica. Review of literature and report of 41 additional cases. Surg., Gynec. & Obst., 62: 712, 1936. WATSON, W., AND McCARTHY, W. D.: Blood and lymph vessel tumors. A report of 1,056 cases. Surg., Gynec., and Obst., 71: 569, 1940.
AND
MILTON HELPERN
From The Beth David Hospital, New York, N. Y.
Primary tumors of the epididymis whether benign or malignant are very rare. In 1936 Thompson in a review of the literature and a report of 41 additional cases of tumors of the spermatic cord, epididymis and testicular tunic mentions only 3 cases of lymphangioma of the epididymis recorded in the literature. In 1939, 3 years later, Charache reviewing the literature on primary tumors of the epididymis listed 6 cases of lymphangioma including 1 of his own. Of the 54 cases of primary neoplasm of the epididymis which he recorded 22 were benign and of these 6 were lymphangiomata. Spivak in 1935 listed 11 cases of leiomyoma of the epididymis. Falconer reported a similar case in 1938, and Milner and Gilbert in 1939 described a case of bilateral leiomyomata of the epididymis in a 60 year old man in whom the Aschheim-Zondek test was negative. Halpert in 1941 reported a case of mixed leiomyoma of the epididymis as the first recorded example of such a tumor. The tumor he described was removed from a 60 year old Negro in whom swelling of the epididymis was noted 2 months after a suprapubic prostatectomy and bilateral vasectomy. The tumor was excised 3 months after the swelling was first noted and measured 3 by 2 by 1.5 cm. Grossly it had the appearance of a leiomyoma. Microscopically it was composed of a mixture of smooth muscle tissue and lymph capillaries imbedded in a connective tissue stroma. In this paper we are reporting a tumor of the epididymis similar to the one described by Halpert. CASE REPORT
A 57-year old white man, and a paper cutter by trade, stated that about 4 weeks before his admission to the Beth David Hospital on May 25, 1941, a ream of paper had slipped from his hands and struck him in the right groin. At that time, he experienced a sharp pain at that site, but the pain gradually subsided until 3 days before his admission to the hospital, when he became aware of a swelling of the right testicle accompanied by a sense of discomfort and dull pain in that organ. Physical examination disclosed a small, smooth, firm non-tender nodule about the size of a hazelnut in the right epididymis. The testicle was normal in size, shape and consistency. The spermatic cord on that side was also smooth and normal on palpation. There was no evidence of any inguinal hernia and no enlargement of the inguinal lymph nodes. The left testicle, epididymis and spermatic cord were normal. A preoperative diagnosis of possible traumatic right epididymitis was made, and the possibility of a neoplasm of the epididymis was also considered. On admission to the hospital the blood count revealed 4,950,000 erythrocytes, a hemoglobin of 97 per cent, 7900 leucocytes of which 66 per cent were poly104
MIXED LEIOMYOMA AND .LYMPHANGIOMA OF EPIDIDYMIS
105
morphonuclears, 3·2 per cent lymphocytes and 2 per cent monocytes. The Kline and Wassermann tests on the blood were negative. An Aschheim-Zondek test was carried out on a sample of urine obtained in the morning before operation on May 26. At the operation, the head of the epididymis, which was replaced by a tumor nodule, was resected by one of (S. M.) and submitted for rapid frozen section and histologic examination, and the diagnosis of a neoplasm was confirmed. An orchidectomy was then performed. When the epididymis was first removed, several cubic centimeters of clear straw-colored fluid were evacuated from the tunica vaginalis. Culture of this fluid was negative. The tunica vaginalis was not adherent to the testicle. A portion of the ductus deferens was also excised. Following the operation, the patient made an uneventful recovery and was discharged from the hospital ten days after admission. The Friedman modification of the Aschheim-Zondek test on the patient's urine, which was carried out on 1 rabbit, was positive, suggesting that the tumor of the epididymis would prove malignant. This was not borne out by the pathologic examination. Subsequently, three additional Aschheim-Zondek tests were performed during the post-operative period on June 4, June 30 and December 18, respectively. These subsequent tests were all negative. It is difficult to evaluate the results of the positive test in this case, although the animals used for all tests were obtained from a reliable source. Nevertheless, the possibility that a non-virgin female rabbit was injected, cannot be eliminated. The specimen first submitted for rapid frozen section was a globular tumor mass, measuring 18 mm. in diameter, replacing the head of the epididymis. The tumor was smooth, firm and elastic, and encapsulated. The resected surface by which it was attached to the pole of the testis measured 18 mm. in diameter. On section, the cut surface of the tumor nodule was gray in color and trabeculated, in appearance very much like that of a leiomyoma. The body and tail of the epididymis and the testis were submitted separately; the former measured 2 cm. in length and 5 mm. in diameter. The testis was normal in size and measured 4.3 by 3.2 by 2.8 cm. The tunica albuginea was smooth and beneath the site of the resected tumor nodule it was slightly thickened. There was a thin layer of freshly extravasated blood in the testicular tissue beneath this portion of the tunica albuginea. The appendices of the epididymis and of the testis were not found. Section of the testis revealed a normal lobular pattern. The structures in the spermatic cord were normal. Sections of the tumor nodule replacing the head of the epididymis revealed an encapsulated overgrowth of interconnecting fascicles of smooth muscle fibers intermingled with collagen fibers. This fibromuscular stroma was interspersed unevenly with many small closely placed, variable-sized clear spaces, separated from each other by thin delicate cytoplasmic walls containing flattened nuclei (fig. 1). At first glance, the pattern of these clear spaces suggested adipose tissue. On close examination, some of these spaces were found to contain small numbers of mononuclear leucocytes. Frozen sections of the tissue stained with Sudan III did not reveal any fat in these spaces, many of which were quite
106
SAMUEL MALISOFF AND MILTON HELPERN
large. '\¥here these spaces were numerous and large, the tissue had a honeycombed appearance. In other areas, where the vascular spaces were smaller, the supporting reticulum and endothelial lining cells were more readily made out. Some of the vascular spaces were formed intracellularly, first appearing as vacuoles in the cytoplasm of the endothelial cells. In such cells, the nuclei were more vesicular in character. Connective tissue reticulum was variable in amount and there were small scattered interstitial foci of lymphocytes. The tumor was enclosed in a thin fibrous tissue capsule. The fibromuscular charac-
FIG. 1. X 60
ter of the stroma was confirmed by the Van Gieson stain. No recognizable ducts or other tissue of the epididymis were found in any of the sections of the tumor. Sections of the testis beneath the site where the tumor of the epididymis was removed revealed slight fibrous thickening of the tunica of the tunica albuginea, and slight fresh hemorrhage beneath the tunica incident to the operation. The lymph vessels in the tunica are slightly dilated. The seminiferous tubules were fairly well preserved and active spermatogenesis was found in many of the
MIXED LEIOMYOMA AND LYMPHANGIOMA OF EPIDIDYMIS
107
tubules. Some atrophic and some dilated tubules were also found. The lymph vessels in the interstitium of the testis were dilated. There was no evidence of any neoplasm within the testis. Sections of the body and tail of the epididymis distal to the tumor revealed ·well-preserved ducts lined by ciliated epithelium. Many of these ducts contained spermatozoa. The lymph spaces in the epididymis were also slightly dilated, especially in the periphery, and some of the ducts of the epididymis were dilated.
Fm. 2. X 200
Diagnosis: The neoplasm replacing the head of the epididymis is most adequately diagnosed as a mixed leiomyoma and lymphangioma. DISCUSSION
Several papers describing cases of lymphangioma of the epididymis discuss the various theories of the histogcnesis of the tumor. A mechanical theory assumes that the lymphangioma occurs as a result of an obstruction to the flow of lymph resulting from inflammation of the lymphatics and lymph nodes,
108
SAMUEL MALISOFF AND MILTON HELPERN
:alteration in the perilymphatic tissues or obliteration of lymph channels as the result of traumatic injury, surgery or thrombosis. However, in none of the cases observed have such mechanical factors been satisfactorily demonstrated nor has there been any clinical evidence of lymph stasis. The heteroplastic theory, which assumes an origin of the elements of a lymphatic tumor from other types of tissue by metaplastic transformation, does not have much evidence to support it. The theory which is most generally accepted considers the lymphangioma to be a new growth derived from pre-existing lymphatic tissues. It is also possible that the lymphangioma has an embryonal basis for its origin. This possibility must certainly be considered in the present case because of the intimate association of the lymphangiomatous elements with the leiomyomatous portions of the tumor. Despite the history of trauma elicited in this case the pathologic findings do not lend any support to the theory that trauma is a factor in the causation of the tumor. There is nothing in the gross or histologic findings to indicate anything of a traumatic character in and around the site of the lesion. The tumor itself does not contain any elements that one would expect to encounter during an exaggerated reparativc process. There is no evidence of any old hemorrhage or scar tissue which might be interpreted as a residue and as an indicator of a previous traumatic injury. Except for the lymphangiomatous component of the tumor, the growth in no way differs from that of the ordinary leiomyoma so frequently encountered in the uterus and not infrequently found in the gastro-intestinal tract, sites where trauma has never been considered a causal factor. The positive Aschheim-Zondek test obtained preoperatively in 1 rabbit is difficult to evaluate. The tumor does not contain any elements comparable to those found in tumors which do give rise to the gonadatropic hormone. The possibility that the rabbit used was not a virgin cannot be eliminated. In the case reported by Milner and Gilbert the Aschheim-Zondek test was negative. SUMMARY
A second case of mixed leiomyoma and lymphangioma of the epididymis is reported. The pathologic findings, despite the history of a traumatic injury to the groin 4 weeks before the recognition of the tumor, do not suggest that trauma was a factor in the development of the tumor.
5 W. 86th St., New York, N. Y. REFERENCES CHARACHE, H.: Lymphangioma of the epididymis. Review of primary tumors of epididymis. Urol. and Cutan. Rev., 43: 663, 1939. FALCONER, B.: Zur Kenntniss der primaren N ebenhodengeschwulste. Ztsch. f. Krebsforsch., 48: 243, 1938. HALPERT, B.: Mixed leiomyoma and lymphangioma of the epididymis: report of a case. J. Urol., 45: 536, 1941. MARCANDIER ET THOMAS: Lymphangioma of the epididymis. Bull. de L'assoc. franc. p. l'Etude du cancer, 19: 126, 1930.
MIXED LEIOMYOMA AND LYMPHANGIOMA OF EPIDIDYMIS
109
MILNER, W. A., AND GILBERT, J.B.: Unique case of bilateral leiomyomata of epididymis. J. Urol., 42: 619, 1939. RIGANO-lRRERA, D.: On a case of simple circumscribed lymphangioma of the tail of the epididymis. Arch. Ital. di chir., 13: 552, 1925. ScALFr, A.: Cases of simple lymphangioma of epididymis. Boll. d. soc. med.-chir., Pavia, 49: 1053, 1935. ScALFI, A.: Benign tumors of the epididymis, Ann. ital. di chir., 16: 81, 1936. SPIVAK, A. H.: Leiomyoma of epididymis. Report of a case and review of literature. J. Urol., 34: 122, 1935. THOMPSON, G. J. : Tumors of the spermatic cord, epididymis and testicular tunica. Review of literature and report of 41 additional cases. Surg., Gynec. & Obst., 62: 712, 1936. WATSON, W., AND McCARTHY, W. D.: Blood and lymph vessel tumors. A report of 1,056 cases. Surg., Gynec., and Obst., 71: 569, 1940.